Foreword
Myositis Through the Ages
Bibliography
Preface
Contents
Contributors
1: Introduction to Myositis
Introduction
What Is Myositis?
Historical Perspective
The Classification of Myositis
Conclusion
References
2: Myositis Basics/Who Gets Myositis
Introduction
Prevalence and Incidence of Myositis and Its Subtypes
Age, Gender, Racial/Ethnic and Geographical Differences
Risk of Myositis in Family Members of IIM Patients
The Role of Environmental and Genetic Factors in the Development of Myositis
Environmental Risks: The Role of Noninfectious Risk Factors
Environmental Risks: The Role of Infectious Agents
Genetic Risk Factors in Idiopathic Inflammatory Myopathies
Conclusion
References
3: Evaluating the Patient with Suspected Myositis
Introduction
History
Demographic Considerations
Pattern and Progression of Muscle Weakness
Extra-Muscular Features of Myositis
Social, Family, and Medication Assessment
Physical Examination
Laboratory Evaluation
Imaging Studies
Specialized Tests
Electromyography and Nerve Conduction Study
Muscle Biopsy
Conclusion
References
4: Asymptomatic HyperCKemia
Introduction
Etiology of Asymptomatic HyperCKemia
Problems with the âNormalâ Laboratory CK
Physical Activity, Exercise, and CK Levels
Non-neuromuscular Etiologies of Asymptomatic HyperCKemia
Macro CK
Statins
Endocrine Disorders
Neuromuscular Etiologies of Asymptomatic HyperCKemia
Idiopathic HyperCKemia
Diagnostic Yield of Muscle Biopsy and EMG/NCS in Asymptomatic HyperCKemia
Conclusion: Diagnostic Approach to Asymptomatic HyperCKemia
References
5: Clinical Features of Myositis: Muscular Manifestations
Introduction
Patterns of Muscle Weakness
Myalgia Versus Weakness
Muscle Examination
Dysphagia and Dysphonia
Muscle Damage and Steroid Myopathy
Red Flags for Myositis Diagnosis
Disease Course
Conclusion
References
6: Clinical Features of Myositis: Skin Manifestations
Introduction
Description of Individual DM Rashes
Calcinosis Cutis and Other Uncommon Rashes
Rashes Associated with Clinically Amyopathic Dermatomyositis
Autoantibody Association of the Dermatomyositis Rashes
Differential Diagnosis
References
7: Clinical Features of Myositis: Lung Manifestations
Introduction
Asymptomatic Lung Involvement
Chronic Cough
Rapidly Progressive ILD
Conclusion
References
8: Clinical Features of Myositis: Cardiac Manifestations
Introduction
Pathophysiology
Clinical Manifestations
Subclinical Heart Involvement
Diagnostic Modalities
Management
Prognosis
Conclusions and Recommendations
References
9: Clinical Features of Myositis: Arthritis, Raynaud Phenomenon, Constitutional
Introduction
Arthritis and Arthralgia
Rheumatoid Factor (RF) and Anti-CCP Positivity in Idiopathic Inflammatory Myopathies
Radiographic Findings of Arthritis
Raynaud Phenomenon (RP)
Constitutional Symptoms
Fever
Fatigue
Myalgia
Conclusion
References
10: Clinical Features of Myositis: Juvenile Dermatomyositis
Introduction
Muscle Disease
Skin Disease
Extra-muscular Disease Features
Myositis-Specific Autoantibodies
Diagnosis of Juvenile Dermatomyositis
References
11: Making the Diagnosis of Myositis: Definition and Classification of Myositis
Introduction
When to Suspect Myositis
Classification Criteria
The EULAR/ACR Myositis Classification Criteria
IBM Diagnostic/Classification Criteria
Conclusion
References
12: Making the Diagnosis of Myositis: Electrodiagnostic Testing
Introduction
Planning the Study
Nerve Conduction Study
Needle Electromyography
Voluntary Contraction: Motor Unit Potential (MUP) Assessment
Recruitment
EMG Findings in Myositis
The Needle Examination Report
Caveats and Correlations
Differential Diagnosis
References
13: Making the Diagnosis of Myositis: Muscle Biopsy and Interpretation
Introduction
Selecting a Muscle Biopsy Site
Processing the Specimen
Interpretation of Muscle Biopsy Findings
How to Read the Muscle Biopsy Report
Dermatomyositis (DM)
Polymyositis (PM)
Inclusion Body Myositis (IBM)
Necrotizing Autoimmune Myopathy
Antisynthetase Syndrome
Steroid and Other Toxic Myopathies
Metabolic Myopathies and PM Mimics
References
14: Making the Diagnosis of Myositis: Skin Biopsy and Interpretation
Introduction
Histologic Findings in Cutaneous Dermatomyositis
Where to Biopsy and When to Biopsy?
Additional Stains Used in the Workup of Suspected Cutaneous Dermatomyositis
Regional/Anatomic and Clinical Variations in Histologic Findings in Cutaneous Dermatomyositis
Histologic Differential Diagnosis
Ancillary Techniques
Conclusion
References
15: Making the Diagnosis of Myositis: Lung Biopsy and Interpretation
Introduction
Histopathologic Patterns of Myositis-Associated Lung Disease
Distinguishing Myositis-Associated Lung Disease from Other Lung Diseases
Association of Myositis Subtypes and Autoantibodies with Histopathologic Features
Prognosis of Myositis-Related Lung Disease
Should I Obtain a Lung Biopsy?
What Kind of Biopsy Should I Ask for?
Conclusion
References
16: Making the Diagnosis of Myositis: Muscle MRI
Introduction
MRI Protocols in IIM
Types of Muscle Magnetic Resonance Imagings
Diagnostic Yield of Magnetic Resonance Imaging
Distribution of Magnetic Resonance Imaging Involvement in IIM Subtypes
Magnetic Resonance Imaging in Guiding Muscle Biopsies
MRI Pattern in Myositis Mimics
Magnetic Resonance Imaging for Disease Activity Versus Damage
Magnetic Resonance Imaging for Treatment Response
Other Advantages of Magnetic Resonance Imaging in Myositis
Functional Magnetic Resonance Imaging Techniques
Muscle Ultrasound
FDG-PET
99mTechnetium Pyrophosphate Scintigraphy
Conclusion
References
17: Making the Diagnosis of Myositis: Laboratory Testing in Myositis
Introduction
Muscle Enzymes: CK, Aldolase, LDH, AST, and ALT
Inflammatory Markers: Ferritin, ESR, CRP, etc
Anti-nuclear Antibodies and Anti-cytoplasmic Pattern in Myositis
Anti-cytoplasmic Pattern in Anti-synthetase Syndrome
Myositis Autoantibodies: When to Order and How to Interpret
References
18: Role of ANA and Myositis Autoantibodies in Diagnosis
Introduction
Autoantibody Detection
Diagnosis
Conclusion
References
19: Role of Myositis Autoantibodies in Management and Prognosis
Introduction
Malignancy Survey
Risk Stratification and Treatment of Interstitial Lung Disease
Refractory Myopathy
References
20: Traditional Myositis Autoantibodies: Synthetase, Mi-2, SRP, Ku, PM-Scl, Ro, U1RNP
Introduction
Antisynthetase Antibodies (Anti-ARS)
Anti-Mi-2
Anti-Signal Recognition Particle Antibodies (Anti-SRP)
Anti-PM-Scl and Anti-Ku
Anti-Ro/SSA, Anti-U1RNP
References
21: Dermatomyositis-Associated Autoantibodies: TIF1-Îł, NXP2, and MDA5
Introduction
Transcription Intermediary Factor 1-Îł (TIF1-Îł)
Nuclear Matrix Protein 2 (NXP2)
Melanoma Differentiation-Associated Gene 5 Protein (MDA5)
References
22: Newly Described Myositis Autoantibodies: HMGCR, NT5C1A, SAE, PUF60
Introduction
Anti-HMGCR
Anti-NT5C1A (Anti-CN1A)
Anti-PUF60
Anti-SAE
Other Emerging MSA/MAAs
References
23: Myositis Mimics: The Differential Diagnosis of Myositis
Introduction
Myositis Mimics: The Differential Diagnosis of Myositis
Identifying and Diagnosing the Metabolic Myopathies
McArdle Disease
Acid Maltase Deficiency
Mitochondrial Myopathies
Identifying and Diagnosing the Muscular Dystrophies
Facioscapulohumeral Muscular Dystrophy
Dysferlinopathy
Calpainopathy
Identifying the Inclusion Body Myositis Patient
Endocrinopathies and Muscle Symptoms
Toxic Myopathies
Amphiphilic Drugs
Antimicrotubular Myopathy
Antiretroviral Agents
Alcohol
Self-Limited Statin-Associated Myopathic Symptoms
Steroid Myopathies
Other Myositis Mimics
Funding
References
24: Immune-Mediated Necrotizing Myopathy (IMNM)
Introduction
Anti-HMGCR Autoantibody-Positive Myopathy, Also Called Statin-Associated Immune-Mediated Necrotizing Myopathy
Anti-SRP Autoantibody-Positive Myopathy
Autoantibody-Negative Immune-Mediated Necrotizing Myopathy
Immune-Mediated Necrotizing Myopathies Associated with Anti-Synthetase Syndrome and Other Connective Tissue Diseases
Cancer-Associated Necrotizing Myopathy
Selected Management Features
Management of Anti-HMGCR Ab+ Myopathy
Anti-SRP+ Myopathy
Conclusion
References
25: Cancer-Associated Myositis
Introduction
Epidemiology
Risk Factors
Autoantibody Associations
Cancer Screening
Role of Tumor Markers in Cancer Screening
Role of Positron Emission Tomography/Computed Tomography (PET/CT) in Cancer Screening
Follow-Up of Cancer-Associated Myositis
Management
References
26: Myositis-Associated Interstitial Lung Disease
Introduction
Case No. 1
Case No. 2
Symptoms
Physical Examination
Signs and Symptoms of Possible Underlying Myositis
Autoantibodies Associated with ILD (See Chaps. 20â21)
Demographics and Clinical Features Associated with ILD
Natural History of ILD
Radiographic Patterns in Myositis-Associated ILD
Strategies for Treating ILD
Pharmacologic Therapy
Nonpharmacologic Therapy
Prognosis of Myositis-Associated ILD
Outcomes and Selection for Lung Transplantation
Other Pulmonary Complications
Conclusion
References
27: Pregnancy in Myositis
Introduction
Pregnancy Effect on the Disease
Disease Effect on Pregnancy (Maternal and Fetal Outcomes)
Anesthetic Management for Cesarean Delivery in Myositis Patients
Clinical and Pathological Correlations
Treatment of Myositis During Pregnancy
Concomitant Medication
Conclusion
References
28: Pathogenesis of Myositis
Introduction
Genetics and Environment
Adaptive Immunity
Role of B and T Cells
Role of Innate Immunity
Non-immune Mechanisms
Vascular Pathology and the Role of Ischemia
Conclusions
References
29: Management Considerations: Pharmacologic Intervention
Introduction
Immunosuppressive Medications
Intravenous Immunoglobulin (IVIg)
Biologic Agents
Other Treatment Considerations
Conclusion
References
30: Management Considerations: Juvenile Dermatomyositis
Introduction
General Approach
Pharmacologic Therapy
Glucocorticoids
Hydroxychloroquine (Plaquenil)
Methotrexate
Cyclosporine
Mycophenolate Mofetil (MMF)
Azathioprine (Imuran)
Cyclophosphamide (Cytoxan)
Intravenous Immunoglobulin (IVIG, Gamunex, Gammagard)
Biological Therapies
B-Cell Depletion
Rituximab (Rituxan)
Anti-T Cell
Abatacept (Orencia)
Anti-cytokines
Tocilizumab (Actemra)
Anti-tumor Necrosis Factor (Anti-TNF)
Infliximab (Remicade)
Etanercept (Enbrel)
Future Therapeutic Targets
Additional Treatments
Stem Cell Transplantation
Physical Therapy and Exercise
Skin Involvement
Calcinosis
Conclusion
References
31: Management Considerations: Refractory Skin Rash and Calcinosis
Introduction
Principles of Management of Cutaneous Dermatomyositis
Differentiate Active Skin Disease from Damage
Target all Organs Involved, Not Just the Skin
Topical Therapy Is Mainly Adjunctive Therapy
Discuss the Importance of Photoprotection
Knowing the Patientâs Dermatomyositis-Specific Antibody May Be Useful
Most Treatment Guidelines for Cutaneous Dermatomyositis Are Expert Opinions
Treatment Ladder for the Inflammatory Erythema of Cutaneous Dermatomyositis
Biologics and Other Agents
Specific Scenarios
Calcinosis
Procedural Options
Calcium Modulators and Vasoactive Medications
Anti-inflammatory Medications
References
32: Management Considerations: Interstitial Lung Disease
Introduction
Specific Agents, Dosage, and Side Effects
Treatment Consideration for Distinct Clinical Scenarios
Monitoring of Therapy
Conclusion
References
33: Role of Exercise in the Management of Myositis
Introduction
The Role of Physical and Occupational Therapy
Physical Therapy
The Anti-inflammatory Effects of Exercise Intervention
Exercise as a Treatment Modality in PM and DM: A Randomized Controlled Trial
Effects of Exercise in PM and DM
Exercise Programs in Inclusion Body Myositis
Exercise Programs in Juvenile Dermatomyositis (JDM)
Introducing Exercise to a Patient
Occupational Therapy
Activities of Daily Living in Myositis
Polymyositis and Dermatomyositis
Inclusion Body Myositis
Occupational Therapy Treatment
Conclusion
References
34: Dietary Considerations in Myositis
Introduction
Gluten Sensitivity and Gluten-Free Diet
Vitamin D Deficiency
Creatine Supplementation
Protein Supplementation
Retinoid Acid
Dietary Statin
Anti-inflammatory Diet and Gut Microbiome
Conclusion
References
35: Distinguishing Disease Activity and Damage in Myositis
Introduction
Muscle Weakness: Activity vs. Damage
Differentiating Activity from Damage Based on Muscle Testing
Dyspnea: Activity vs. Damage
Assessment of the Skin
Assessment of Joints
Conclusions
References
Index